Asymptomatic Advanced Pediatric Papillary Renal Cell Carcinoma Presenting as a Pulmonary Embolus

Paediatric RCC with sarcomatoid variation: a rare entity

“…The most common renal malignancy in childhood is Wilms' tumour with renal cell carcinoma (RCC) being very uncommon accounting for 2-5% of all paediatric renal malignancies [4]. Renal cell carcinoma originates within the epithelium of renal tubules and may be of papillary, clear cell, chromophobe and oncocytoma morphology [5].…”

“…Paediatric RCC is mostly papillary type with high survival rate after treatment [5]. Predictors of poor survival like sarcomatoid differentiation, perivascular and perineural invasion are extremely rare.…”

Pathology, Therapy and Prognosis of Papillary Renal Carcinoma

“…Kidney cancer is the 13th most common malignancy worldwide, with ~ 271 000 new cases . RCC is extremely rare in the pediatric population and accounts for ~ 6% of malignant pediatric tumors (‹ 4% of pediatric renal tumors) [3].…”

“…Papillary RCC (pRCC) comprises ~ 6-18% of kidney tumors in reported series [6]. It is the most common histologic subtype observed in pediatric RCC and it was reported in 18 % of dialysis patients with less than 10 years of treatment [3,7]. Atypical cysts with extensive papillary hyperplasia are often the precursors of papillary renal adenoma and cancer in these patients [8].…”

Image_1.TIF

“…Papillary RCC (PRCC) is the second most common type of RCC, accounting for nearly 18% of RCC (Srigley et al, 2013). In addition, it is the most common histological subtype in pediatric RCC and has been reported in 18% of dialysis patients (Morabito et al, 2010). However, diagnosis, treatment, and prognosis of PRCC are now mostly based on histological features, whose subtyping remains unsatisfactory (Fernandes and Lopes, 2015).…”